[Diffuse familial polyposis]. / Polyposes familiales diffuses.

Familial gastrointestinal polyposis constitutes a multiplication of the principle varieties of common polyps, not only in the colon but also in other parts of the gastrointestinal tract. These are hereditary and familial diseases with autosomal dominant transmission. The genetic defect can also be responsible for associated extra-gastrointestinal lesions. The classification of these familial polyposes is based on the histological features of the polyps. The adenomatous polyposes consist of familial recto-colonic polyposis (FRCP), which is the most frequent form, and Gardner's syndrome. This variety of polyposis selectively involves the recto-colon, but also presents gastric and duodenal lesions. The defining feature of Gardner's syndrome is the extra-gastrointestinal mesenchymal lesions, osteomas, skin tumours and, most importantly, mesenteric fibromatosis which may complicate abdominal operations. The prognosis of FRCP and Gardner's syndrome is determined by the almost obligatory and often multicentric malignant degeneration of the polyps, which justifies routine total proctocolectomy. Peutz-Jeghers' syndrome is syndrome is characterised by hamartomatous polyposis selectively involving the upper part of the small intestine. It generally has a benign course. Similarly, juvenile polyposis consists of benign inflammatory polyps and is accompanied by congenital anomalies in one third of cases. The diagnosis of familial polyposis should be proposed whenever polyps are detected, which appear to be unusual in terms of their number or their distribution. A family survey should be performed in such cases.

[Colonic pseudo-occlusion syndrome. Ogilvie's syndrome Apropos of 22 cases]. / Le syndrome de pseudo-occlusion colique. Syndrome d'Ogilvie. A propos de 22 cas.

The syndrome of pseudo colonic obstruction consists of acute or subacute colonic dilatation which presents the diagnostic and therapeutic problems of any low bowel obstruction. Our experience is based on a series of 22 cases and is similar to data in the literature. There is a specific aetiological context: elderly subjects, vascular and neurological diseases, severe infection, trauma, alcoholism. The exclusion of an organic occlusion is not always an easy diagnosis and may require an exploratory laparotomy. The clinical course is dominated by the risk of diastatic perforation which carries a heavy mortality. In the majority of cases, the colonic peristalsis is restored by early diagnosis, repeated decompression with a rectal catheter, together with general measures of rehydration, control of infection and maintenance of satisfactory haemodynamic conditions. In the other cases, a draining colostomy may be required. The pathophysiological mechanism of this form of colonic ileus remains hypothetical, in which various neurogenic, vascular, toxic or metabolic factors may be involved.

[Severe digestive manifestations of rheumatoid purpura in adults]. / Manifestations digestives sévères du purpura rhumatoide de l'adulte.

The authors report three cases of Scholein-Henoch's purpura in adults which were characterized by atypical severity of the digestive signs which led to laparotomy. In one case, the digestive signs preceded the onset of the cutaneous purpura, which made the diagnosis difficult. The digestive impact was confirmed endoscopically (petechia, ulceration, ulcerated stenosis) in two of the three patients. Damage of the small intestine predominated, as in the literature, but only one patient required resection of the intestine. These observations highlight the importance of endoscopic exploration in cases of abdominal signs combined with Scholein-Henoch's purpura in adults. They also demonstrate the difficulty of evaluating the prognosis and treatment in severe, peritoneo-occlusive forms.

[Isolated ACTH deficiency associated with leukoneutropenia (author's transl)]. / Association d'une leucopénie majeure et d'une insuffisance isolée en ACTH.

The paper describes the case of a 38 year's old alcoholic patient. The investigation of his severe asthenia led to the discovery of an isolated ACTH deficiency: plasma cortisol was less than 1 microgram 100 ml reactivable by tetracosa-peptide beta 1-24. Plasma ACTH was low and remained so after LVD and metyrapone. Persistent leuconeutropenia without myelogram and agar cultures abnormalities was noted. Etiocholanolone test was negative and epinephrine induced a significant rise in blood leucocytes. The authors review the mechanisms of corticoid action on leucopoiesis. After 10 months corticoid treatment, leuconeutropenia improved but was still present suggesting an other underlying mechanism than cortisol insufficiency per se.

[Circumscribed peritonitis of genital origin or gonococcal perihepatitis Fitz-Hugh Curtis syndrome (author's transl)]. / Péritonite localisée d'origine génitale ou pérhépatite gonococcique. Syndrome de Fitz-Hugh Curtis.

Circumscribed peritonitis with perihepatitis resulting from previous female gonococcal infection is now commmonly known as Fitz-Hugh Curtis (FHC) syndrome. Contamination of the peritoneum through the Fallopian tubes and the fact that the gonococcal inflammation (frequency, though not always confirmed) remains superficial with little production of pus explain why the perihepatic lesions are fibrinous, adhesion-forming and tend to subside spontanesouly. The pathophysiological mechanisms, however, are complicated by recent reports of FHC syndrome in males. Beside Neisseria gonorrhoeae, other organisms growing in the genital tract are probably involved. When necessary, coelio-laparoscopy is the method of choice to confirm the diagnosis. The four cases reported here illustrate the various symptomatic and evolutive aspects of the syndrome.